The DATE Association: A Separate Entity or a Further Extension of the VACTERL Association?

Silvia Ceccanti, Paola Midrio, Mario Messina, Girolamo Mattioli, Antonio Appignani, Vito Briganti, Maurizio Cheli, Mario Lima, Denis A Cozzi

Research output: Contribution to journalArticle

Abstract

BACKGROUND: The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we summed up with the mnemonic acronym DATE [D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia].

METHODS: This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes.

RESULTS: The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean.

CONCLUSIONS: The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO.

Original languageEnglish
Pages (from-to)128-134
Number of pages7
JournalJournal of Surgical Research
Volume241
DOIs
Publication statusPublished - Sep 2019

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    Ceccanti, S., Midrio, P., Messina, M., Mattioli, G., Appignani, A., Briganti, V., Cheli, M., Lima, M., & Cozzi, D. A. (2019). The DATE Association: A Separate Entity or a Further Extension of the VACTERL Association? Journal of Surgical Research, 241, 128-134. https://doi.org/10.1016/j.jss.2019.03.054